What is Ichthyosis Hystrix?

Ichthyosis hystrix is a form of epidermal nevus. Epidermal nevus is the clinical term for disorders in which the hyperkeratosis (thickening of the outer layer of skin) occurs in linear and swirling patterns within typically larger areas of uninvolved, normal skin. They are mosaic skin disorders caused by several different genes (for more information see FIRST’s information on epidermal nevi). The term ‘mosaic’ indicates that only some of the cells in the body harbor the mutation. In some instances, mosaic disorders are heritable (for more information, request FIRST’s publications, Ichthyosis: the Genetics of its Inheritance and epidermal nevi).

As more information for our “Patient Perspective” section of Ichthyosis Hystrix becomes available, we will provide updates. Please refer to a Clinician’s Perspective for Ichthyosis Hystrix Syndrome.

This information is provided as a service to patients and parents of patients who have ichthyosis. It is not intended to supplement appropriate medical care, but instead to complement that care with guidance in practical issues facing patients and parents. Neither FIRST, its Board of Directors, Medical & Scientific Advisory Board, Board of Medical Editors nor Foundation staff and officials endorse any treatments or products reported here. All issues pertaining to the care of patients with ichthyosis should be discussed with a dermatologist experienced in the treatment of their skin disorder.